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World J Hepatol. Jul 27, 2014; 6(7): 464-476
Published online Jul 27, 2014. doi: 10.4254/wjh.v6.i7.464
Juvenile autoimmune hepatitis: Spectrum of the disease
Giuseppe Maggiore, Silvia Nastasio, Marco Sciveres
Giuseppe Maggiore, Silvia Nastasio, Department of Clinical and Experimental Medicine, Pediatric Gastroenterology, University Hospital of Pisa, 56127 Pisa, Italy
Marco Sciveres, Pediatric Hepatology and Pediatric Liver Transplantation, UPMC Ismett, 90100 Palermo, Italy
Author contributions: All authors equally contributed to this paper.
Correspondence to: Giuseppe Maggiore, MD, Department of Clinical and Experimental Medicine, Pediatric Gastroenterology, University Hospital of Pisa, Via Roma 67, 56127 Pisa, Italy. giuseppe.maggiore@med.unipi.it
Telephone: + 39-50-992639 Fax: + 39-50-993044
Received: November 7, 2013
Revised: May 19, 2014
Accepted: May 29, 2014
Published online: July 27, 2014
Processing time: 259 Days and 17.3 Hours
Abstract

Juvenile autoimmune hepatitis (JAIH) is a progressive inflammatory liver disease, affecting mainly young girls, from infancy to late adolescence, characterized by active liver damage, as shown by high serum activity of aminotransferases, by elevated immunoglobulin G levels, high titers of serum non organ-specific and organ-specific autoantibodies, and by interface hepatitis on liver biopsy. It is a multifactorial disease of unknown etiology in which environmental factors act as a trigger in genetically predisposed individuals. Two types of JAIH are identified according to the autoantibody panel detected at diagnosis: AIH-1, characterized by the presence of anti-smooth muscle antibody and/or antinuclear antibody and AIH-2, by anti-liver-kidney microsomal antibody type 1 and/or by the presence of anti-liver cytosol type 1 antibody. Epidemiological distribution, genetic markers, clinical presentation and pattern of serum cytokines differentiate the two types of AIH suggesting possible pathogenetic mechanisms. The most effective therapy for AIH is pharmacological suppression of the immune response. Treatment should be started as soon as the diagnosis is made to avoid severe liver damage and progression of fibrosis. The aim of this review is to outline the most significant and peculiar features of JAIH, based largely on our own personal database and on a review of current literature.

Keywords: Juvenile autoimmune hepatitis; Autoimmune hepatitis; Autoantibodies; Autoimmune liver disease; Chronic hepatitis; Acute liver failure

Core tip: Juvenile autoimmune hepatitis is an inflammatory liver disease affecting mainly young girls from infancy to late adolescence, characterized by active liver damage, elevated immunoglobulin G levels, high titers of serum non organ-specific and organ-specific autoantibodies, and interface hepatitis on liver biopsy. Two types are identified according to the autoantibody panel, with differences in the epidemiological distribution, genetic markers and clinical presentation. The most effective therapy for autoimmune hepatitis is pharmacological suppression of the immune response. Treatment should be started as soon as the diagnosis is made to avoid severe liver damage and progression of fibrosis.